Endocardial cushion defect

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2]

Endocardial cushion defects is a congenitial disorder most commonly associated with Down’s syndrome. They are part of AV canal disorder resulting due to either genetic mutations or alteration in growth hormone in fetus leading to cardiac malformation during embryogenesis. Based on the anatomical features and their impact on physiology, endocardial cushion defect may be classified into complete, partial, intermediate, transitional, and intermediate forms. AV canal connects the atria to the ventricles. At four to five weeks of gestation, the superior and inferior endocardial cushions of the common AV canal fuse. Results in the formation of the mitral and tricuspid valve and the AV septum. Any failure of fusion results in endocardial cushion defect. Pathophysiology of endocardial cushion defects depends upon level of left to right shunting and degree of shunting. Incompetent AV valves in endocardial cushion defect results in regurgitation. ndocardial cushion defects are transmitted in families as an autosomal dominant. The characteristic pattern of genetic mutation has been attributed to trisomy 21 and Down syndrome. The blowing holosystolic murmur of endocardial cushion defects must be distinguished from mitral regurtitaion, tricuspid regurgitation and a ventricular septal defect. Though ECG and chest x-ray may share some common features, echocardiography can be efficiently used for an accurate diagnosis. The prevalence of endocardial cushion defect is approximately 300 to 400 per 1000,000 live births. Certain factors might increase risk of developing endocardial cushion defect include down syndrome, rubella, alcohol consumption during pregnancy, gestational diabetes, smoking during pregnancy. Routine fetal ultrasound during prenatal care can detect endocardial cushion defects. Diagnostic findings on fetal ultrasound suggestive of endocardial cushion defect include large defect at the crux of the heart that involves the atrial and ventricular septa and a large common AV valve. If left untreated, majority of patients with endocardial cushion defect may progress to develop life threatening conditions. Common complications of endocardial cushion defect include dilatation of heart, pulmonary hypertension, respiratory tract infections , and heart failure. Surgical mortality rate of patients with partial endocardial cushion defect is approximately 0.6%. For complete cushion defect the surgical mortality rate is 2.5-9%. Prognosis of endocardial cushion defect is generally good with treatment. However, some children might develop valvular and rhythm disorders after surgical correction. Echocardiography can be helpful in the diagnosis of endocardial cushion defect. Findings on an echocardiography diagnostic of endocardial cushion defect include diastolic movement of the mitral valve with paradoxical motion of the interventricular septum. Other findings on echocardiography include absence of the interventricular septum and right ventricular dilation. The majority of patients with endocardial cushion defect are asymptomatic. Symptoms of endocardial cushion defect include chronic upper respiratory tract infections, pneumonia, and poor growth attributable to feeding difficulties. Patients with endocardial cushion defect may have a positive history of difficulty with crying, frequent pauses during feeding and nasal flaring. Volume overload of the right side of heart can lead to right heart failure that may present with symptoms of swelling of the extremities, difficulty breathing and signs such as hepatomegaly and an elevated jugular venous pulse. On cardiovascular examinations there is a fixed splitting of second heart sound. Also, a systolic ejection murmur that is attributed to the increased flow of blood through the pulmonic valve can be heard. Patients with endocardial cushion defect may have polycythemia on CBC, which is usually due to cyanosis. Presence of a superior axis directed more towards the left is the most characterstic feature of endocardial cushion defects on electrocardiogram. Other findings on a ECG suggestive of endocardial cushion defect include prolongation of PR interval and right ventricular enlargement. The management of endocardial cushion defect depends upon the type of defect, underlying etiology and associated cardiac conditions. Surgical correction of defective valve holds the the mainstay of treatment for endocardial cushion. Medical management provides supportive care in preparing the patient for surgery. The management of endocardial cushion defect depends upon the type of defect, underlying etiology and associated cardiac conditions. Surgical correction of defective valve holds the the mainstay of treatment for endocardial cushion. Medical management provides supportive care in preparing the patient for surgery. Effective measures for the secondary prevention of endocardial cushion defects include annual cardiology evaluation, routine neurological screening, infective endocarditis prophylaxis, and risk assessment during pregnancy.

Based on the anatomical features and their impact on physiology, endocardial cushion defect may be classified into complete, partial, intermediate, transitional, and intermediate forms.

AV canal connects the atria to the ventricles. At four to five weeks of gestation, the superior and inferior endocardial cushions of the common AV canal fuse. Results in the formation of the mitral and tricuspid valve and the AV septum. Any failure of fusion results in endocardial cushion defect. Pathophysiology of endocardial cushion defects depends upon level of left to right shunting and degree of shunting. Incompetent AV valves in endocardial cushion defect results in regurgitation. There is a strong association between endocardial cushion defects and Down syndrome

The most common cause of endocardial cushion defect is genetic mutations. Endocardial cushion defects are transmitted in families as an autosomal dominant. The characteristic pattern of genetic mutation has been attributed to trisomy 21 and Down syndrome.

The blowing holosystolic murmur of endocardial cushion defects must be distinguished from mitral regurtitaion, tricuspid regurgitation and a ventricular septal defect. Though ECG and chest x-ray may share some common features, echocardiography can be efficiently used for an accurate diagnosis.

The prevalence of endocardial cushion defect is approximately 300 to 400 per 1000,000 live births. There is no racial predilection to endocardial cushion defects and it affects men and women equally.

There are no established risk factors for endocardial cushion defects. However, certain factors might increase risk of developing endocardial cushion defect include down syndrome, rubella, alcohol consumption during pregnancy, gestational diabetes, smoking during pregnancy.

Routine fetal ultrasound during prenatal care can detect endocardial cushion defects. Diagnostic findings on fetal ultrasound suggestive of endocardial cushion defect include large defect at the crux of the heart that involves the atrial and ventricular septa and a large common AV valve.

If left untreated, majority of patients with endocardial cushion defect may progress to develop life threatening conditions. Common complications of endocardial cushion defect include dilatation of heart, pulmonary hypertension, respiratory tract infections , and heart failure. Surgical mortality rate of patients with partial endocardial cushion defect is approximately 0.6%. For complete cushion defect the surgical mortality rate is 2.5-9%. Prognosis of endocardial cushion defect is generally good with treatment. However, some children might develop valvular and rhythm disorders after surgical correction.

Echocardiography can be helpful in the diagnosis of endocardial cushion defect. Findings on an echocardiography diagnostic of endocardial cushion defect include diastolic movement of the mitral valve with paradoxical motion of the interventricular septum. Other findings on echocardiography include absence of the interventricular septum and right ventricular dilation.

The majority of patients with endocardial cushion defect are asymptomatic. Symptoms of endocardial cushion defect include chronic upper respiratory tract infections, pneumonia, and poor growth attributable to feeding difficulties. Patients with endocardial cushion defect may have a positive history of difficulty with crying, frequent pauses during feeding and nasal flaring.

Volume overload of the right side of heart can lead to right heart failure that may present with symptoms of swelling of the extremities, difficulty breathing and signs such as hepatomegaly and an elevated jugular venous pulse. On cardiovascular examinations there is a fixed splitting of second heart sound. Also, a systolic ejection murmur that is attributed to the increased flow of blood through the pulmonic valve can be heard.

Patients with endocardial cushion defect may have polycythemia on CBC, which is usually due to cyanosis.

Presence of a superior axis directed more towards the left is the most characterstic feature of endocardial cushion defects on electrocardiogram. Other findings on a ECG suggestive of endocardial cushion defect include prolongation of PR interval and right ventricular enlargement.

There are no x-ray findings associated with endocardial cushion defect. However, an x-ray may be helpful in the general screening of endocardial cushion defect which demonstrates cardiac enlargement and increased pulmonary vascular markings.

Echocardiography can be helpful in the diagnosis of endocardial cushion defect. Findings on an echocardiography diagnostic of endocardial cushion defect include diastolic movement of the mitral valve with paradoxical motion of the interventricular septum. Other findings on echocardiography include absence of the interventricular septum and right ventricular dilation.

There are no CT scan findings associated with endocardial cushion defect.

There are no MRI findings associated with endocardial cushion defect.

There are no other imaging findings associated with endocardial cushion defect.

There are no other diagnostic studies associated with endocardial cushion defect.

The management of endocardial cushion defect depends upon the type of defect, underlying etiology and associated cardiac conditions. Surgical correction of defective valve holds the the mainstay of treatment for endocardial cushion. Medical management provides supportive care in preparing the patient for surgery.

There are no recommended therapeutic interventions for the management of endocardial cushion defects.

The management of endocardial cushion defect depends upon the type of defect, underlying etiology and associated cardiac conditions. Surgical correction of defective valve holds the the mainstay of treatment for endocardial cushion. Medical management provides supportive care in preparing the patient for surgery.

There are no established measures for the primary prevention of endocardial cushion defect.

Effective measures for the secondary prevention of endocardial cushion defects include annual cardiology evaluation, routine neurological screening, infective endocarditis prophylaxis, and risk assessment during pregnancy.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2]

Based on the anatomical features and their impact on physiology, endocardial cushion defect may be classified into complete, partial, intermediate, transitional, and intermediate forms.

Based on the anatomical features and their impact on physiology, endocardial cushion defect may be classified into complete, partial, intermediate, transitional, and intermediate forms.^[1]

Type	Pathophysiology	Characteristics
Complete AV canal	Complete failure to fuse of superior and inferior cushions	Primum ASD Posterior VSD Common AV valve
Partial AV canal	Incomplete fusion of the superior and inferior cushions
Transitional AV canal	Similar physiology of a partial AV canal defect Dense chordal attachments to the ventricular septum lead to small insignificant ventricular shunting Delineation of distinct left and right AV valve orifices	Large primum defect Mitral valve cleft Inlet VSD
Intermediate AV	Bridging tongue of tissue divides the common AV valve into two distinct orifices.	Large primum atrial Inlet ventricular septal defects.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2]

AV canal connects the atria to the ventricles. At four to five weeks of gestation, the superior and inferior endocardial cushions of the common AV canal fuse. Results in the formation of the mitral and tricuspid valve and the AV septum. Any failure of fusion results in endocardial cushion defect. Pathophysiology of endocardial cushion defects depends upon level of left to right shunting and degree of shunting. Incompetent AV valves in endocardial cushion defect results in regurgitation. There is a strong association between AV canal defects and Down syndrome

• AV canal connects the atria to the ventricles.^[1]
• At four to five weeks of gestation, the superior and inferior endocardial cushions of the common AV canal fuse.^[2]
• Results in the formation of the mitral and tricuspid valve and the AV septum.

Pathophysiology of endocardial cushion defects depends upon

• Level of left to right shunting
• Degree of shunting

	Level of left-to-right shunting	Pathophysiology
Complete defect	At the atrial and ventricular levels	↑ pulmonary blood flow Leading to heart failure and ↑ PVR
Partial defect	At the level of the primum atrial septal defect	Volume overload of the right atrium and ventricle and pulmonary vasculature. Pulmonary artery pressures are usually normal. Minimal symptoms
Transitional defect	Shunting is minimal Due to a small and restrictive ventricular septal defect.	Similar to those of partial canal defect

• AV valves are incompetent in endocardial cushion defect resulting in regurgitation
  • In complete defects, regurgitation through LV to LA or RV to RA.
  • In partial defects, most of the regurgitation is from LV to LA through the cleft in the anterior mitral valve leaflet.

• There is a strong association between AV canal defects and Down syndrome.^[3]

• Chromosome 21 has been designated an AV canal critical region.
• Trisomy 21 have an AV canal defect, usually the complete form

Common cardiac conditions associated with endocardial cushion defect include:^[4][5]

• Tetralogy of Fallot
• Transposition of the great arteries
• Patent ductus arteriosus
• Coarctation of the aorta
• Absent atrial septum
• Persistent left superior vena cava
• Anomalous pulmonary venous connection

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2]

The most common cause of endocardial cushion defect is genetic mutations. Endocardial cushion defects are transmitted in families as an autosomal dominant. The characteristic pattern of genetic mutation has been attributed to trisomy 21 and Down syndrome.

• The most common cause of endocardial cushion defect is genetic mutations.^[1]
• Endocardoal cushion defects are transmitted in families as an autosomal dominant.
• The characteristic pattern of genetic mutation has been attributed to trisomy 21 and Down syndrome. Other common causes include
  • Deletion of 8p
  • Partial 10q monosomy
  • Partial 13q monosomy
• Alteration of growth factor beta and platelet-derived growth factor in fetus during embryogenesis leads to cardiac tissue malformation.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Varun Kumar, M.B.B.S.; Lakshmi Gopalakrishnan, M.B.B.S.; Mohammed A. Sbeih, M.D. [3]; Yamuna Kondapally, M.B.B.S[4]

The blowing holosystolic murmur of endocardial cushion defects must be distinguished from mitral regurtitaion, tricuspid regurgitation and a ventricular septal defect. Though ECG and chest x-ray may share some common features, echocardiography can be efficiently used for an accurate diagnosis.

All the three cardiac conditions have holosystolic murmur on auscultation. But they can be differentiated by characteristics of the murmur detailed below:^[1]

Mitral Regurgitation	Tricuspid Regurgitation	VSD
The murmur in mitral regurgitation is high pitched and best heard at the apex with diaphragm of the stethoscope with patient in the lateral decubitus position. Left ventricular function can be assessed by determining the apical impulse. A normal or hyperdynamic apical impulse suggests good ejection fraction and primary mitral regurgitation. A displaced and sustained apical impulse suggests decreased ejection fraction and chronic and severe mitral regurgitation.	Can be best heard over the fourth intercostal area at left sternal border. The intensity can be accentuated following inspiration (Carvallo’s sign) due to increased regurgitant flow in right ventricular volume. Tricuspid regurgitation is most often secondary to pulmonary hypertension. Primary tricuspid regurgitation is less common and can be due to bacterial endocarditis following IV drug use, Ebstein’s anomaly, carcinoid disease, or prior right ventricular infarction.	The holosystolic murmur can be best heard over the left third and fourth intercostal spaces and along the sternal border. When the shunt becomes reversed (“Eisenmenger’s syndrome“), the murmur may be absent and S2 can become markedly accentuated and single.

The above three cardiac conditions can also be differentiated more definitively using echocardiography where the echogenicity of blood flow across the defective valves or septum can be visualized and the severity can be quantified.

Mitral regurgitation must be differentiated from the following:^{[2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24]}

Diseases	History	Symptoms	Physical Examination	Murmur	Diagnosis				Other Findings
					ECG	CXR	Echocardiogram	Cardiac Catheterization
Mitral Stenosis	Age ( Mitral annular calcification in older patients) Rheumatic fever Endocarditis	Dyspnea on exertion Paroxysmal nocturnal dyspnea Orthopnea New onset atrial fibrillation	Mitral facies Heart murmur Jugular vein distension Apical impulse displaced laterally or not palpable Diastolic thrill at the apex Signs of heart failure in severe cases	Diastolic murmur Low pitched Opening snap followed by decrescendo-crescendo rumbling murmur Best heard with the bell of the stethoscope at apex at end-expiration in left lateral decubitus position Intensity increases after a valsalva maneuver, after exercise and after increased after load (eg., squatting, isometric hand grip)	P mitrale Atrial fibrillation: No P waves and irregularly irregular rhythm Right axis deviation Right ventricular hypertropy: Dominant R wave in V1 and V2	Straightening of the left border of the heart suggestive of enlargement of the left atrium Double right heart border (Enlarged left atrium and normal right atrium) Prominent left atrial appendage Splaying of subcarinal angle (>120 degrees) Calcification of mitral valve Kerley B lines	Reduced valve leaflet mobility Valve calcification Doming of mitral valve Valve thickening Enlargement of left atrium	Right heart catheterization: Pulmonary capillary wedge pressure (left atrial pressure) Left heart catheterization: Pressures in left ventricle Determines the gradient between the left and right atrium during ventricular diastole (marker of the severity of mitral stenosis)	Hemoptysis (heart failure) Ortner’s syndrome
Mitral Regurgitation	CAD MI Rheumatic fever Endocarditis Mitral valve prolapse Cardiomyopathy Radiation therapy Trauma	Palpitations Symptoms of heart failure in severe cases	Palpation Brisk carotid upstroke and hyperdymanic carotid impulse on palpation Apical impulse is displaced to left S3 and a palpable thrill Auscultation Murmur	Holosystolic murmur High pitched, blowing Radiates to axilla Best heard with the diaphragm of the stethoscope at apex in left lateral decubitus position Intensity increases with hand grip or squatting Decrease in intensity on standing or valsalva maneuver	P mitrale in lead II Increased QRS voltage Right axis deviation Atrial fibrillation	Acute MR Kerley B lines No enlargement of cardiac silhouette Chronic MR Enlarged cardiac silhouette Straightening of left heart border Splaying of subcarinal angle Calcification of mitral annulus Double right heart border	Enlargement of left atrium and ventricle Identify valve abnormality Valve calcification Severity of regurgitation	Grading of MR is done with left ventriculography	Decompensated and acute MR may lead to heart failure
Atrial septal defect	Frequent respiratory or lung infections Dyspnea Tiring when feeding (Infants) Shortness of breath on exertion Palpitations Swelling of feet	Shortness of breath Fatigue Failure to thrive Swelling of feet and abdomen (Right heart failure) Palpitations Respiratory infections	Inspection Precordial bulge Precordial lift Palpation Right ventricular impulse Pulmonary artery pulsations Thrill Auscultation Murmur	Midsystolic (ejection systolic) murmur Widely split, fixed S2 Upper left sternal border	Normal Prolonged PR interval Right bundle branch block ECG findings varies according to the underlying type of ASD	Increased pulmonary markings Cardiomegaly Triangular appearance of heart Schimitar sign	Gold standard test for diagnosis of atrial septal defect (for more information click Atrial septal defect echocardiography)	Defect size Pulmonary venous return Pulmonary vascular resistance Pulmonary artery hypertension	Asymptomatic until later part of their life May be associated with migraine with aura
Left Atrial Myxoma	Dyspnea Orthopnea Pulmonary edema Hyperpigmentation of skin and endocrine activity Cerebral embolism	Symptoms may mimic mitral stenosis	Skin Signs of an embolic phenomenon Raynaud’s phenomenon Swelling Clubbing Auscultation: Lung: Fine crepitations Heart: Characteristic “tumor plop”	Early diastolic sound as “tumor plop” Low frequency diastolic murmur may be heard if the tumor obstructing mitral valve	Often normal	Often normal Rare findings: cardiomegaly Left atrial enlargement tumor calcification etc.,	Initial and most useful diagnostic study For more information click Myxoma echocardiography or ultrasound	Useful to detect vascular supply of the tumor by the coronary arteries	Associated with Carney complex (genetic predisposition)
Prosthetic Valve Obstruction	History of valve replacement Systemic embolism	Shortness of breath Fatigue	Ausculation Muffling of murmur	Muffling or disappearance of prosthetic sounds Appearance of new regurgitant or obstructive murmur			Degree of stenosis Assess thrombus size and location Differentiate between thrombus, pannus and vegetations		Causes: Thrombus Pannus formation
Cor Triatriatum	Dyspnea on exertion Recent onset of congestive heart failure	Dsypnea on exertion Orthopnea Tachypnea Palpitations Growth failure	Auscultation Murmur Other findings Signs of heart failure	Diastolic murmur with loud P2 No opening snap or a loud S1	Non specific but may have Right axis deviation Right atrial enlargement Right ventricular hypertrophy	Normal cardiac silhouette Hemodynamic changes similar to mitral stenosis (non specific findings)	Direct visualization of membrane through the atrium +/- visualization of accessory chamber	Normal left ventricular hemodynamic profile with a trans atrial gradient	Types Cor triatriatum sinistrum Cor triatriatum dextrum
Congenital Mitral Stenosis	Respiratory distress shortly after birth Recurrent severe pulmonary infections Other associated congenital cardiovascular anamolies Atrial fibrillation	Infants: Exhaustion and sweating on feeding Rapid breathing Failure to thrive Pulmonary infections Chronic cough Older patients: Dyspnea Orthopnea Paroxysmal nocturnal dyspnea Peripheral edema Fatigue	Auscultation Murmur Other findings Signs of heart failure	Mild-Moderate Loud S1 Loud P2 Low frequency diastolic murmur best heard at the apex Severe Soft S1 Loud pulmonic component of S2 with minimal respiratory splitting of S2 Holodiastolic murmur with presystolic accentuation best heard at the apex Early diastolic murmur of pulmonic valve regurgitation	Sharp P waves in leads I and II Inversion of P wave in lead III Marked Q waves in leads II and III	Left atrial dilation Moderate enlargement of right heart Pulmonary venous congestion Esophageal compression	Reduced valve leaflet mobility Left atrial size Severity of mitral stenosis		Very rare condition
Supravalvular Ring Mitral Stenosis	Other associated congenital heart defects Fatigue Frequent respiratory infections Failure to thrive Poor feeding Precocious congestive heart failure	Shortness of breath Tachypnea Dyspnea Nocturnal cough Heamoptysis Syncope	Auscultation: Lungs: Fine, crepitant rales and rhonchi or wheezes may be present Heart: Murmur	An apical mid diastolic murmur with presystolic accentuation No opening snap The murmur is more prominent if associated with VSD or PDA		Left atrial and ventricular enlargement Alveolar edema	Supramitral ring: Associated with normal mitral valve apparatus Intramitral ring: Hypomobility of the posterior leaflet Reduced interpapillary muscle distance Reduced chordal length Dominant papillary muscle Hypoplastic mitral annulus (Difficult to visualize membrane <1mm in size)	Persistently elevated pulmonary venous pressures Increased pulmonary artery pressure	Types Supramitral Intramitral It is attached between the opening of the atrial appendage and the mitral annulus which helps in differentiating with Cor triatriatum sinister. Intramitral type is associated with shone complex

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ;Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2]

The prevalence of endocardial cushion defect is approximately 300 to 400 per 1000,000 live births. There is no racial predilection to endocardial cushion defects and it affects men and women equally.

• The prevalence of endocardial cushion defect is approximately 300 to 400 per 1000,000 live births.^[1][2]

• There is no racial predilection to endocardial cushion defects.

• Endocardial cushion defects affects men and women equally.^[3]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2]

There are no established risk factors for endocardial cushion defects. However, certain factors might increase risk of developing endocardial cushion defect include down syndrome, rubella, alcohol consumption during pregnancy, gestational diabetes, smoking during pregnancy.

There are no established risk factors for endocardial cushion defects. However, certain diseases/factors might increase risk of developing endocardial cushion defect include:^[1][2]

• Down syndrome
• Rubella
• Alcohol consumption during pregnancy
• Gestational diabetes
• Smoking during pregnancy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2]

Routine fetal ultrasound during prenatal care can detect endocardial cushion defects. Diagnostic findings on fetal ultrasound suggestive of endocardial cushion defect include large defect at the crux of the heart that involves the atrial and ventricular septa and a large common AV valve.

• During prenatal care routine fetal ultrasound can help in making an in-utero diagnosis of endocardial cushion defect.^[1][2]
• Diagnostic findings on fetal ultrasound suggestive of endocardial cushion defect include:
  • Large defect at the crux of the heart that involves the atrial and ventricular septa,
  • Large common AV valve
• Abnormalities detected on fetal ultrasound should be followed by a fetal echocardiography for confirmation due to the poor sensitivity of fetal ultrasound.^[3]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2]

If left untreated, majority of patients with endocardial cushion defect may progress to develop life threatening conditions. Common complications of endocardial cushion defect include dilatation of heart, pulmonary hypertension, respiratory tract infections , and heart failure. Surgical mortality rate of patients with partial endocardial cushion defect is approximately 0.6%. For complete cushion defect the surgical mortality rate is 2.5-9%. Prognosis of endocardial cushion defect is generally good with treatment. However, some children might develop valvular and rhythm disorders after surgical correction.

• If left untreated, majority of patients with endocardial cushion defect may progress to develop life threatening heart failure and pulmonary hypertension due to excessive pulmonary blood flow.

• Common complications of endocardial cushion defect include:^[1][2]
  • Dilatation of heart
  • Pulmonary hypertension
  • Respiratory tract infections
  • Heart failure

• Prognosis of endocardial cushion defect is generally good with treatment. However, some children might develop valvular and rhythm disorders after surgical correction.^[3]
• Prognositic factors include:
  • Degree of preoperative pulmonary vascular disease
  • Amount of residual AV valve regurgitation
• Protected pulmonary vascular bed with mild AV regurtiation is associated with good outcome, while the contrary holds the bad prognosis.
• Surgical mortality rate of patients with partial endocardial cushion defect is approximately 0.6%. For complete cushion defect the surgical mortality rate is 2.5-9%

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